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Pemphigoid gestationis


Other Names for this Disease

  • Gestational pemphigoid
  • Herpes gestationis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My daughter has been diagnosed with pemphigoid gestationis 5 weeks after giving birth.  Do you have any information that can provide information on this rare autoimmune disease? Are there any clinical studies currently being performed?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pemphigoid gestationis (PG)?

Pemphigoid gestationis (PG), also known as gestational pemphigoid or herpes gestationis, is an autoimmune skin disorder associated with pregnancy. Signs and symptoms of the disorder typically present during the second or third trimester of pregnancy, but it may occur at any time during pregnancy or anywhere up to six weeks after giving birth. Although the signs and symptoms are variable, a red, itchy rash with blisters is common. The rash may occur in different parts of the body, but usually develops near the belly button and/or the arms and legs. The condition is caused by the production of antibodies that attack the person's body, leading to damage and consequently blister formation. Treatment generally involves systemic corticosteroids.[1] 
Last updated: 8/11/2010

What are the signs and symptoms of pemphigoid gestationis?

The signs and symptoms of PG are variable. The first sign of PG tends to be an extremely itchy red rash that begins in the  abdominal area and trunk, usually involving the belly button. The rash quickly progresses to blisters that may also affect the palms and soles. The other areas of the body are usually spared. The blisters usually heal without leaving a scar.[2]
Last updated: 8/11/2010

What causes pemphigoid gestationis?

PG is caused by the production of an antibody that belongs to a specific class of antibodies called IgG1. This antibody attaches to a specific protein called bullous pemphigoid antigen. The antigen-antibody complex, through a series of reactions, results in the release of certain molecules by special cells called eosinophils. The molecules, which are normally released in response to inflammation or infection, cause damage in the layers of the skin, leading to symptoms of PG such as blister formation.[1][2]
Last updated: 8/11/2010

How is pemphigoid gestationis diagnosed? When is it typically diagnosed?

PG is confirmed by performing a biopsy to determine the presence of deposits of a particular protein in a specific area of the skin.[2]

Approximately 75 percent of cases of PG occur during pregnancy; however, up to 25 percent occur in the postpartum period. Some have suggested that the postpartum cases are actually mild cases of HG that were undiagnosed during pregnancy.[2]
Last updated: 8/11/2010

What treatment is available for pemphigoid gestationis?

Patients with HG typically respond well to low-dose treatment with oral corticosteroids like prednisone. In more resistant cases, higher doses or oral corticosteroids might be needed. Up to about 75 percent of patients experience a spontaneous resolution or improvement during the late third trimester, and treatment might be discontinued. Since post-delivery flare-ups of HG are common, some individuals who had experienced improvement may require an increase in corticosteroids doses in anticipation. For more difficult-to-manage cases treatment may includes topical corticosteroids, oral antihistamines, and immunosuppressants.[3][2]
Last updated: 8/11/2010

Does pemphigoid gestationis affect the fetus? If so, how?

Mothers with PG may be at an increased risk of having a baby that is small-for-gestational age or preterm. Some have suggested that mild placental insufficiency may be the cause of these risks. Sonograms of the baby during the third trimester might be appropriate and used to monitor fetal growth and development. Another risk associated with maternal PG is the placental trasnder of the PG antibody, resulting in neonatal PG; this occurs in about 5 to 10 percent of babies.  The blisters resolve spontaneously without scarring over a period of weeks as the maternal PG antibodies are cleared by the baby. Babies with neonatal PG should be monitored for bacterial infections of the lesions. Early treatment can prevent progression to systemic infection.[2] 
Last updated: 8/11/2010

Are there any long-term complications associated with pemphigoid gestationis?

In general, there are no long-term complications associated with PG. However, an association between PG and other autoimmune diseases like Graves' disease has been reported; therefore, it is performance of immediate and periodic screening tests of thyroid function is recommended. Some women will have persistent HG or recurrent flares lasting weeks or months following birth. Additionally, it is important to recognize that women who have had PG are at an increased risk of postpartum flare-ups, if they use oral contraceptives within 6 months of giving birth.[2]
Last updated: 8/11/2010

Does pemphigoid gestationis recur in future pregnancies?

Yes. Approximately 95 percent of women have PG with future pregnancies and lesions may be more severe, appear earlier during the pregnancy, and last longer after delivery.[2]
Last updated: 8/11/2010

References
  • Flangini Cobo M, Giuli Santi C, Wakisaka Maruta C, Aoki V. Pemphigoid Gestationis: Clinical and Laboratory Evaluation. Clinics. 2009;
  • Specific Dermatoses of Pregnancy. In: Gabbe SG, Niebyl JR, Simpson JL. Gabbe: Obstetrics: Normal and Problem Pregnancies, 5th ed. Philadelphia: Churchill Livingstone Elsevier; 2007;
  • Doiron P, Pratt M. Antepartum intravenous immunoglobulin therapy in refractory pemphigoid gestationis: case report and literature review. J Cutan Med Surg. 2010 Jul-Aug;
Other Names for this Disease
  • Gestational pemphigoid
  • Herpes gestationis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.